Electrophysiological signs of peripheral nerve dysfunction in progressive myoclonus epilepsy.
Electrophysiological findings were analysed in a group of 24 patients with progressive myoclonus epilepsy (PME) without Lafora bodies. Denervation activity in needle EMG and diminution of motor and sensory responses pointed out a mild axonal degeneration. We observed a significant slowing of motor and sensory conduction velocities in all the limb nerves examined, but distal motor latencies were not significantly increased. H-reflex latency of the posterior tibial nerve was prolonged. These results yielded the suggestion that there is a systemic peripheral nerve membrane dysfunction in PME.
D000293:Adolescent; D000328:Adult; D004576:Electromyography; D004831:Epilepsies, Myoclonic / Q000503:physiopathology*; D006801:Humans; D008875:Middle Aged; D009431:Neural Conduction; D010525:Peripheral Nerves / Q000503:physiopathology*; D011930:Reaction Time / Q000502:physiology; D012023:Reflex, Monosynaptic; D012881:Skin Temperature